大疱性类天疱疮诊疗专家共识（2025版）

doi:10.35541/cjd.20240622

中华皮肤科杂志 ›› 2025, Vol. 58 ›› Issue (5): 405-415.doi: 10.35541/cjd.20240622

大疱性类天疱疮诊疗专家共识（2025版）

中国医师协会皮肤科医师分会中华医学会皮肤病学分会治疗学组中国医疗保健国际交流促进会皮肤医学分会国家皮肤与免疫疾病临床医学研究中心中国罕见病联盟皮肤罕见病专业委员会

中国医师协会皮肤科医师分会中华医学会皮肤病学分会治疗学组中国医疗保健国际交流促进会皮肤医学分会国家皮肤与免疫疾病临床医学研究中心中国罕见病联盟皮肤罕见病专业委员会

收稿日期:2024-11-14 修回日期:2025-01-17 发布日期:2025-04-30
通讯作者: 左亚刚；晋红中 E-mail:zuoyagang@263.net; jinhongzhong@263.net
基金资助:
国家重点研发计划（2022YFC3601800）；中央高水平医院临床科研业务费（2022-PUMCH-B-092）；北京自然科学基金（7232118）；北京市临床重点专科项目；国家临床重点专科建设项目

Diagnosis and treatment of bullous pemphigoid: an expert consensus statement （2025 edition）

China Dermatologist Association; Treatment Group, Chinese Society of Dermatology; Dermatology Branch of China International Exchange and Promotive Association for Medical and Health Care; National Clinical Research Center for Dermatologic and Immunologic Diseases; Rare Skin Diseases Committee, China Alliance for Rare Diseases

China Dermatologist Association; Treatment Group, Chinese Society of Dermatology; Dermatology Branch of China International Exchange and Promotive Association for Medical and Health Care; National Clinical Research Center for Dermatologic and Immunologic Diseases; Rare Skin Diseases Committee, China Alliance for Rare Diseases

Received:2024-11-14 Revised:2025-01-17 Published:2025-04-30
Contact: Zuo Yagang; Jin Hongzhong E-mail:zuoyagang@263.net; jinhongzhong@263.net
Supported by:
National Key R&D Program of China （2022YFC3601800）; National High Level Hospital Clinical Research Funding （2022-PUMCH-B-092）; Beijing Municipal Natural Science Foundation （7232118）; Beijing Key Clinical Specialty Construction Project; National Key Clinical Specialty Project of China

摘要/Abstract

摘要： 【摘要】大疱性类天疱疮（BP）是一种主要影响老年人的自身免疫性表皮下大疱性皮肤病，临床表现为全身或局部皮肤出现紧张性水疱和大疱，部分患者危及生命。BP的发病机制与抗BP180和BP230抗体密切相关，通过补体激活系统和非补体系统等途径致病。本共识涵盖BP的临床特征、诊断标准、治疗方案及合并症管理，特别强调个体化治疗的重要性。在诊断方面，需结合临床表现、组织病理、直接免疫荧光及血清学检查，以提高诊断准确性。治疗上，除传统的糖皮质激素和免疫抑制剂，推荐生物制剂等新型药物，并根据病情严重程度制定个性化方案。同时，共识还关注BP合并症如糖尿病、肾病、恶性肿瘤等的管理，为临床医生提供全面指导，提升BP治疗效果和患者的生活质量。

关键词: 类天疱疮, 大疱性, 诊断, 治疗学, 共识, 生物制剂

Abstract: 【Abstract】 Bullous pemphigoid （BP） is an autoimmune subepidermal blistering skin disease that primarily affects the elderly. Clinically, it is characterized by tense blisters and bullae on the local or whole body skin, which can be life-threatening in some patients. The pathogenesis of BP is closely related to anti-BP180 and anti-BP230 antibodies, which cause BP through pathways such as complement activation systems and non-complement systems. This consensus covers clinical characteristics, diagnostic criteria, treatment options, and management of comorbidities associated with BP, with a particular emphasis on the importance of individualized treatment. In terms of diagnosis, a comprehensive approach integrating clinical manifestations, histopathological features, direct immunofluorescence and serological testing results is proposed to improve diagnostic accuracy. In terms of treatment, in addition to traditional glucocorticoids and immunosuppressive agents, new drugs such as biological agents are also recommended, and individualized treatment regimens are formulated according to the disease severity. Additionally, the consensus also pays attention to the management of BP-related comorbidities such as diabetes, kidney disease, and malignant tumors, providing comprehensive guidance for clinicians to improve the treatment outcomes and quality of life of BP patients.

Key words: Pemphigoid, bullous, Diagnosis, Therapeutics, Consensus, Biological agents

中国医师协会皮肤科医师分会中华医学会皮肤病学分会治疗学组中国医疗保健国际交流促进会皮肤医学分会国家皮肤与免疫疾病临床医学研究中心中国罕见病联盟皮肤罕见病专业委员会. 大疱性类天疱疮诊疗专家共识（2025版）[J]. 中华皮肤科杂志, 2025,58(5):405-415. doi:10.35541/cjd.20240622

China Dermatologist Association, Treatment Group, Chinese Society of Dermatology, Dermatology Branch of China International Exchange and Promotive Association for Medical and Health Care, National Clinical Research Center for Dermatologic and Immunologic Diseases, Rare Skin Diseases Committee, China Alliance for Rare Diseases. Diagnosis and treatment of bullous pemphigoid: an expert consensus statement （2025 edition）[J]. Chinese Journal of Dermatology, 2025, 58(5): 405-415.doi:10.35541/cjd.20240622

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大疱性类天疱疮诊疗专家共识（2025版）

Diagnosis and treatment of bullous pemphigoid: an expert consensus statement （2025 edition）

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